Association between recurrent de novo 22q11.2 microdeletions and schizophrenia represented a shift in our understanding of the genetic architecture of schizophrenia (SCZ), highlighting the role that rare and highly penetrant mutations play in the disease risk (1,2). This view has been strengthened recently by the identification of a widespread role of chromosomal microdeletions and microduplications (copy-number variants or CNVs) in determining susceptibility to schizophrenia and other psychiatric disorders (3-5). Individuals with 22q11.2 microdeletions have specific behavioral impairments and exhibit a spectrum of deficits in cognitive abilities linked to activity in the hippocampus and prefrontal cortex, such as measures of attention, working memory and executive function. Up to one third of children with the microdeletion develop schizophrenia or schizoaffective disorder in adolescence or early adulthood accounting for 1-2% of sporadic schizophrenia cases (2). Understanding how the genes disrupted by this microdeletion contribute to the emergence of the psychiatric and cognitive phenotypes associated with this genomic imbalance would provide important clues for the pathogenesis of SCZ and can guide future analysis of other CNVs that cause psychiatric disorders (6,7).